Research considerations in the evaluation of minimally invasive pancreatic resection (MIPR).

The IHPBA/AHPBA-sponsored 2016 minimally invasive pancreatic resection (MIPR) conference held on April 20th, 2016 included a session designed to evaluate what would be the most appropriate scientific contribution to help define the increasing role of MIPR internationally. Participants in the conference reviewed the assessment of numerous pertinent scientific designs including randomized controlled trial (RCT), pragmatic international RCT, registry-RCT, non-RCT with propensity matching, and various types of clinical registries including those aiming to create a quality improvement data system or a learning health care system. The strengths and weaknesses of each of these designs, the status of trials which are currently recruiting patients, and pragmatic considerations were evaluated.

Novel SIL1 mutations cause cerebellar ataxia and atrophy in a French-Canadian family.

Two French-Canadian sibs with cerebellar ataxia and dysarthria were seen in our neurogenetics clinic. The older brother had global developmental delay and spastic paraplegia. Brain MRIs from these two affected individuals showed moderate to severe cerebellar atrophy.

Teaching neuroimages: hypomyelinating leukodystrophy with hypodontia due to POLR3B: look into a leukodystrophy's mouth.

An 18-year-old German woman presented with progressive cerebellar ataxia since early childhood, delayed cognitive development, and hypogonadotropic hypogonadism. MRI demonstrated diffuse cerebral hypomyelination, cerebellar atrophy, and thin corpus callosum; X-ray revealed persistent milk teeth and hypoplastic crowns and roots (figure), indicative of 4H syndrome (hypomyelination, hypodontia, hypogonadotropic hypogonadism). POLR3B sequencing(1) revealed 2 compound heterozygous mutations (C527R [C.

Mutations of POLR3A encoding a catalytic subunit of RNA polymerase Pol III cause a recessive hypomyelinating leukodystrophy.

Leukodystrophies are a heterogeneous group of inherited neurodegenerative disorders characterized by abnormal white matter visible by brain imaging. It is estimated that at least 30% to 40% of individuals remain without a precise diagnosis despite extensive investigations. We mapped tremor-ataxia with central hypomyelination (TACH) to 10q22.

Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma.

Purpose: To report radiological and pathological response to neo-adjuvant radiotherapy for extremity and trunk soft-tissue sarcomas.

Materials/methods: Fifty patients were identified retrospectively. All patients had MRI imaging pre and post neo-adjuvant external beam radiotherapy.

Predicting glomerular filtration rate in heart transplant recipients using serum creatinine-based equations with cimetidine.

In this study we evaluated the use of cimetidine on the performance of glomerular filtration rate (GFR) estimating equations and 24-hour creatinine clearance (24-hour CrCl) against radionuclide GFR in 43 heart transplant recipients with stable renal function. Pearson correlation coefficients for the 24-hour CrCl, Cockcroft-Gault, Nankivell and Modification of Diet in Renal Disease study (MDRD) equations without cimetidine were 0.76, 0.

Hypofractionated stereotactic radiotherapy for low grade glioma at McGill University: long-term follow-up.

Small, well-defined, unresectable low-grade gliomas are attractive targets for stereotactic irradiation. Fractionated stereotactic irradiation of these targets has the theoretical benefit of increased normal tissue sparing beyond that provided by the physical characteristics of stereotactic radiosurgery. From July 1987 to November 1992, 21 patients were treated for low-grade glioma at our institution using a hypofractionated regimen of stereotactic radiotherapy.